* If you want to update the article please login/register
In both ventral and parietotemporal lobes at early photographs, decreased uptake in both basal ganglia, both frontal and parietotemporal lobes, as well as reduced presynaptic dopamine transporter binding in both ventral and pariettemporal lobes, and posterior putamen at late images. CIT PET/CT and 99mTc-ECD SPECT images in Huntington's disease revealed similar results.
Source link: https://doi.org/10.1016/j.radcr.2022.03.109
GSE3621's gene expression profile was retrieved from the gene expression omnibus. Differentially expressed genes from the R6/1 transgenic mouse model of Huntington's disease and controls at various time points were tested by the limma package in R. Kyoto's encyclopedia of genes and genomes. Huntington's disease is characterized by frequent gene expression changes at various time points. Huntington's disease pathogenesis may be influenced by several key genes, including Usp18, Oasl2, and Rtp4.
Source link: https://doi.org/10.31083/j.jin.2019.04.1176
Huntington's disease is an autosomal dominant hereditary neurodegenerative disease characterized by progressive dystonia, chorea, and psychological or psychiatric disorders. MicroRNAs in Huntington's disease may interact with various transcription factors; dysregulated microRNAs may be linked to the Cytosine deoxynucleotide-Guanine ribonucleotide length and Huntington's disease progression and severity; This research explores the role of microRNAs in Huntington's disease pathogenesis by bioinformatics analysis. We discovered a total of nine differentially expressed microRNAs by reviewing data from the Gene Expression Omnibus database. Homo sapiens -miR-144-3p, the microRNA integrated regulatory network, delivered the most long non-coding RNAs, including X-inactive specific transcript and taurine upregulated gene 1. In addition, 59 Gene Ontology terms and eight enrichment pathways were discovered by analyzing the target genes of hsa-miR-196a-5p and hsa-microRNA-10b-5p.
Source link: https://doi.org/10.31083/j.jin.2020.04.203
YAP activation first between neuronal ectoderm and nonneuronal ectoderm, and later between epidermis and neural crest, implying that YAP involvement may raise the risk of BMP4 stimulation and thus influence ectodermal specification at this developmental stage. To investigate the correlation between signaling and disease, we used isogenic mutant neuruloids to investigate the relationship between signaling and the disease. HD neuruloids show ectopic activation of gene targets of YAP gene expression, as well as pharmacological reduction of YAP's transcriptional activity, which may help to partially restore the HD phenotype.
Source link: https://doi.org/10.7554/eLife.73075
Usually, medication-refractory symptoms and severe chorea are in the early stages of Huntington's disease gait impairments and severe chorea. The long-term effects on gait in HD of physiotherapy are not well-established, according to ICF-based education post-globus pallidus deep brain stimulation. Physiotherapy has long been recognized as a vital component of HD care. After GPi-DBS, we have presented here a case study of a 56-year-old woman with HD on the modern stage and severe chorea medication-refractory. After GPi-DBS intervention and a ICF-based physiotherapy service, Our patient improved substantially HD motor symptoms and quality of life. The patient's motor profile had been assessed during the pre-operative period with objective outcomes measures used to measure gait. ConclusionPeriodically assessing function and disability using outcome enhancements may have helped clinicians make informed decisions about DBS, medication changes, and coaching physiotherapists to personalize the ICF-based intervention.
Source link: https://doi.org/10.3389/fresc.2022.849333
Moreover, we also showed that deranged intracellular Ca 2+ signaling sensitizes YAC128 MSNs to glutamate-induced excitotoxicity in comparison to wild type MSNs. In experiments with these mouse models, we also observed abnormal neuronal Ca 2+ signaling in neurons from spinocerebellar ataxia 2 and spinocerebellar ataxia 3 mouse models, proving that therapy with dantrolene, a ryanodine receptor antagonist and clinically relevant Ca 2+ signaling stabilizer, was neuroprotective. MSN cultures in YAC128 MSN cultures resulted in increased Ca 2+ release from intracellular stores in YAC128 MSN cultures, relative to WT MSN cultures. Results Our results also point to RyanR inhibitors and Ca 2+ signaling stabilizers for HD and other polyqexpansion disorders as potential therapeutic targets for HD and other polyQ-expansion disorders.
Source link: https://doi.org/10.1186/1750-1326-6-81
Mutant huntingtin protein was previously shown to reduce BDNF gene expression and axonal transport of BDNF. In live neurons, we performed imaging experiments to show endogenous Htt and BDNF mRNA co-localization in fixed cells and co-trafficking of BDNF 3'UTR mRNA in cultured cortical neurons. Conclusions In cultured neurons and sections of the rat cortex, we discovered BDNF mRNA linked to Htt and neuronal RNA granules, which are regulatory units for monitoring RNA transport and local translation.
Source link: https://doi.org/10.1186/1750-1326-5-22
Here we established temporal and spatial indices of gait dynamics in a mouse model of PD and a mouse model of HD. The inability of some mice treated with 3NP to demonstrate coordinated gait was due to hind leg impairment, but forelimb gait dynamics remained stable. We also examined gait dynamics in a mouse model of amyotrophic lateral sclerosis to see if gait disturbances related to motor neuron disease or 3NP, drugs affecting the basal ganglia. In the SOD1 G93A transgenic model of ALS in comparison to wild-type control mice, Gait variability was not increased in the SOD1 G93A transgenic model of ALS. Conclusions The distinct features of gait and gait variability in the MPTP model of Parkinson's disease and the 3NP model of Huntington's disease may indicate impairment of specific neural pathways involved.
Source link: https://doi.org/10.1186/1743-0003-2-20
Huntington's Disease is an autosomal dominant genetic disorder in which neuronal tissue degenerates. Mutant huntingtin is produced by HD genes that contain an increased number of glutamine codons within the first exon, and this expansion leads to the production of a protein that misfolds. According to new studies, mutant Htt can nucleate protein aggregation and interfere with a variety of common cell functions. HDG was an excellent inhibitor of aggregation of a fusion protein, composed of a mutant Htt fragment and green fluorescent protein, according to subsequent testing in a cell-based assay. This oligonucleotide can also help cells survive in PC12 cells overexpressing a mutant Htt fragment fusion gene. Single-stranded DNA oligonucleotides capable of generating stable G-quartets can prevent aggregation of the mutant Htt fragment protein.
Source link: https://doi.org/10.1186/1471-2202-7-65
Coenzyme Q10 and minocycline's effects on mouse models of Huntington's disease have resulted in conflicting findings regarding their effectiveness in behavioral tests.
Source link: https://doi.org/10.1371/journal.pone.0009793
* Please keep in mind that all text is summarized by machine, we do not bear any responsibility, and you should always check original source before taking any actions